Steroid-resistant nephrotic syndrome: a persistent challenge for pediatric nephrology

被引:25
作者
Dogra, Samriti [1 ]
Kaskel, Frederick [2 ]
机构
[1] Connecticut Childrens Med Ctr, Div Pediat Nephrol, Dept Pediat, 282 Washington St, Hartford, CT 06095 USA
[2] Childrens Hosp, Albert Einstein Coll Med, Montefiore Med Ctr, Div Pediat Nephrol,Dept Pediat, Bronx, NY USA
关键词
Steroid-resistant nephrotic syndrome; Focal segmental glomerulosclerosis; Minimal change disease; Genetic testing; Glomerular filtration barrier; Podocyte; FOCAL SEGMENTAL GLOMERULOSCLEROSIS; PLASMINOGEN-ACTIVATOR RECEPTOR; CLINICAL-TRIAL; GLOMERULAR-PERMEABILITY; UROKINASE RECEPTOR; IDIOPATHIC FSGS; MUTATIONS; SERUM; PROTEINURIA; RITUXIMAB;
D O I
10.1007/s00467-016-3459-5
中图分类号
R72 [儿科学];
学科分类号
100202 ;
摘要
Steroid-resistant nephrotic syndrome remains a challenge to treat, but various efforts are underway to better understand the pathogenesis and improve patient outcomes. This review provides an update on the newer advances in understanding the molecular etiologies for a variety of podocyte abnormalities, potential circulating factors that may initiate and sustain the steroid-resistant state, genetic mutations, and precision medicine treatment modalities in this continuously perplexing disorder.
引用
收藏
页码:965 / 974
页数:10
相关论文
共 51 条
[1]   PLASMAPHERESIS REDUCES PROTEINURIA AND SERUM CAPACITY TO INJURE GLOMERULI IN PATIENTS WITH RECURRENT FOCAL GLOMERULOSCLEROSIS [J].
ARTERO, ML ;
SHARMA, R ;
SAVIN, VJ ;
VINCENTI, F .
AMERICAN JOURNAL OF KIDNEY DISEASES, 1994, 23 (04) :574-581
[2]   Rituximab in patients with the steroid-resistant nephrotic syndrome [J].
Bagga, Arvind ;
Sinha, Aditi ;
Moudgil, Asha .
NEW ENGLAND JOURNAL OF MEDICINE, 2007, 356 (26) :2751-2752
[3]   Donor splice-site mutations in WT1 are responsible for Frasier syndrome [J].
Barbaux, S ;
Niaudet, P ;
Gubler, MC ;
Grunfeld, JP ;
Jaubert, F ;
Kuttenn, F ;
Fekete, CN ;
SouleyreauTherville, N ;
Thibaud, E ;
Fellous, M ;
McElreavey, K .
NATURE GENETICS, 1997, 17 (04) :467-470
[4]   THE EFFECT OF ADRENOCORTICOTROPHIC HORMONE ON CHILDREN WITH THE NEPHROTIC SYNDROME .2. PHYSIOLOGIC OBSERVATIONS ON DISCRETE KIDNEY FUNCTIONS AND PLASMA VOLUME [J].
BARNETT, HL ;
FORMAN, CW ;
MCNAMARA, H ;
MCCRORY, WW ;
RAPOPORT, M ;
MICHIE, AJ ;
BARBERO, G .
JOURNAL OF CLINICAL INVESTIGATION, 1951, 30 (02) :227-235
[5]  
Benigni A, 2014, NEW ENGL J MED, V370, P1261, DOI 10.1056/NEJMc1400502
[6]   Hereditary nephrotic syndrome: a systematic approach for genetic testing and a review of associated podocyte gene mutations [J].
Benoit, Genevieve ;
Machuca, Eduardo ;
Antignac, Corinne .
PEDIATRIC NEPHROLOGY, 2010, 25 (09) :1621-1632
[7]   uPAR: A versatile signalling orchestrator [J].
Blasi, F ;
Carmeliet, P .
NATURE REVIEWS MOLECULAR CELL BIOLOGY, 2002, 3 (12) :932-943
[8]   Serum Soluble Urokinase-Type Plasminogen Activator Receptor Levels and Idiopathic FSGS in Children: A Single-Center Report [J].
Bock, Margret E. ;
Price, Heather E. ;
Gallon, Lorenzo ;
Langman, Craig B. .
CLINICAL JOURNAL OF THE AMERICAN SOCIETY OF NEPHROLOGY, 2013, 8 (08) :1304-1311
[9]   NPHS2, encoding the glomerular protein podocin, is mutated in autosomal recessive steroid-resistant nephrotic syndrome [J].
Boute, N ;
Gribouval, O ;
Roselli, S ;
Benessy, F ;
Lee, H ;
Fuchshuber, A ;
Dahan, K ;
Gubler, MC ;
Niaudet, P ;
Antignac, C .
NATURE GENETICS, 2000, 24 (04) :349-354
[10]   Association of Histologic Variants in FSGS Clinical Trial with Presenting Features and Outcomes [J].
D'Agati, Vivette D. ;
Alster, Joan M. ;
Jennette, J. Charles ;
Thomas, David B. ;
Pullman, James ;
Savino, Daniel A. ;
Cohen, Arthur H. ;
Gipson, Debbie S. ;
Gassman, Jennifer J. ;
Radeva, Milena K. ;
Moxey-Mims, Marva M. ;
Friedman, Aaron L. ;
Kaskel, Frederick J. ;
Trachtman, Howard ;
Alpers, Charles E. ;
Fogo, Agnes B. ;
Greene, Tom H. ;
Nast, Cynthia C. .
CLINICAL JOURNAL OF THE AMERICAN SOCIETY OF NEPHROLOGY, 2013, 8 (03) :399-406